“Think Zebra” for World Primary Immunodeficiency Week

In partnership with World Primary Immunodeficiency Week celebrated this week (April 22-29th), The Immune Deficiency Foundation is asking everyone to join in to make April Primary Immunodeficiency Awareness Month. People with conditions called primary immunodeficiencies require a regular infusion of IVIG to replace antibodies that their bodies do not produce. IVIG (intravenous immunoglobulin) contains immunoglobulin G and is provided intravenously; a subcutaneous version of immunoglobulin G is also available.

As many as 250,000 people have a primary immunodeficiency, while thousands more go undiagnosed. People with a primary immunodeficiency are more susceptible to infections and illness, which can in turn progress to debilitating diseases. To prevent these chronic infections from causing irreversible damage, early diagnosis is imperative. This need for early diagnosis has fueled organizations such as The Jeffrey Modell Foundation and The Immune Deficiency Foundation in their efforts to educate the public and encourage awareness about these conditions.

If you or someone you know is affected by two or more of the above warning signs, speak to a physician about the possibility of an underlying primary immunodeficiency. Diagnosis is made by history, physical exam, and blood and immunoglobulin level tests.

Once a diagnosis is made, treatment can begin. It is estimated that 70 percent of patients diagnosed with primary immunodeficiency are treated with IVIG. The infusion of IVIG acts as a replacement for what is absent from the patient’s immune system. IVIG is a continuous replacement therapy throughout the life of a patient with primary immunodeficiency.

Ultimately, the goal of treatment for primary immunodeficiency is for those diagnosed with it to lead a full, active life. They should be able to participate fully in school, work, and social activities. The treatment will result in a decrease of the number and severity of infections. The appropriate treatment will produce the least amount of side effects, while being a regimen that works for each patient’s schedule and lifestyle. The constant research, development of new products, and education of pharmacy and care providers are all integral parts of this goal.

Think Zebra The Immune Deficiency Foundation, which is the national patient organization dedicated to persons living with primary immunodeficiency diseases, urges patients and their doctors to “think zebra.”

In medical school, many doctors learn the saying, “when you hear hoof beats, think horses, not zebras” and are taught to focus on the likeliest possibilities when making a diagnosis, not the unusual ones. However, sometimes physicians do need to look for a zebra. Patients with primary immunodeficiency diseases are the zebras of the medical world.

If you have an infection that is: • Severe: requiring hospitalization or intravenous antibiotics • Persistent: won’t completely clear up • Unusual: caused by uncommon organism • Recurrent: keeps coming back • Runs in the family

Published by Dr. Stephen Vogt

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Yes, indeed, when it comes to diagnosis. The problem, once diagnosed and living as a ‘zebra’ is being labelled as ‘complicated and interesting’ leading to endless diagnostics, being pulled-into endless teaching and research activity and difficulties accessing services for the simplest of needs…. ‘can’t treat your ingrowing toenail because you’re too complicated’. Here’s to informed, patient-centred, coordinated care to let the zebras run-free!